title: Congenital middle ear cholesteatoma: experience from 26 surgical cases.
abstract: OBJECTIVE
  We analyzed the clinical features and surgical techniques used in cases of childhood congenital cholesteatoma of the middle ear.
  
  
  METHODS
  We studied 26 patients (26 ears) who underwent surgery for congenital cholesteatoma between January 1998 and December 2009, focusing on the location and type of cholesteatoma, the surgical procedures involved, and the results obtained. Patients with prior otologic procedures were excluded. A 4-stage system was used to grade the cholesteatomas.
  
  
  RESULTS
  The frequency of posterior-quadrant involvement and open-type cholesteatomas increased in the more advanced stages. Second-look operations were performed in 60% of stage III and 75% of stage IV cases; and residual cholesteatomas were found in 20% of stage III and 75% of stage IV cases. Of the cases evaluated both before and after the operation, 100% of stage I and II cases, 86% of stage III cases, and 50% of stage IV cases showed improvement in hearing function.
  
  
  CONCLUSIONS
  The staging system is relatively simple, while accurately reflecting clinical results. However, there are many differences between the anterior and posterior types of congenital cholesteatomas in surgical approach and postoperative progression that are not reflected in the classification systems and require further study. In addition, we reviewed the surgical procedures involved in anterior-quadrant cases, and propose a modified surgical procedure.
authors.affiliation: Department of Otorhinolaryngology-Head and Neck Surgery, Hamamatsu University School of Medicine, Hamamatsu, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Yamatodani
authors.forename: Kunihiro
authors.initials: K
authors.lastname: Mizuta
authors.forename: Kumiko
authors.initials: K
authors.lastname: Hosokawa
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Takizawa
authors.forename: Kenichi
authors.initials: K
authors.lastname: Sugiyama
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Nakanishi
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Mineta
mesh_terms: D000293:Adolescent; D001301:Audiometry, Pure-Tone; D002648:Child; D002675:Child, Preschool; D002781:Cholesteatoma / Q000145:classification / Q000151:congenital* / Q000601:surgery; D018424:Cholesteatoma, Middle Ear / Q000145:classification / Q000151:congenital* / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D013506:Otologic Surgical Procedures / Q000379:methods*; D016896:Treatment Outcome